Pediatric surgery: Decades after pediatric heart surgery, new problems emerging for many patients

Clint Pharr, left, chats with Dr. C. Huie Lin, a congenital and interventional cardiology specialist, at Houston Methodist Hospital. Pharr had his first heart surgery when he was 15 months old to fix a congenital heart defect and a second when he was 33 to fix his pulmonary valve. less

Clint Pharr, left, chats with Dr. C. Huie Lin, a congenital and interventional cardiology specialist, at Houston Methodist Hospital. Pharr had his first heart surgery when he was 15 months old to fix a ... more

Photo: Michael Ciaglo, Staff

Photo: Michael Ciaglo, Staff

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Clint Pharr, left, chats with Dr. C. Huie Lin, a congenital and interventional cardiology specialist, at Houston Methodist Hospital. Pharr had his first heart surgery when he was 15 months old to fix a congenital heart defect and a second when he was 33 to fix his pulmonary valve. less

Clint Pharr, left, chats with Dr. C. Huie Lin, a congenital and interventional cardiology specialist, at Houston Methodist Hospital. Pharr had his first heart surgery when he was 15 months old to fix a ... more

Photo: Michael Ciaglo, Staff

Pediatric surgery: Decades after pediatric heart surgery, new problems emerging for many patients

Born with a congenital heart defect, he underwent surgery in 1982 when he was 15 months old. And all his life he believed doctors had fixed his faulty anatomy. A former high school baseball star, the 35-year-old Houston man competed in mixed martial arts and could bench press 500 pounds.

But three years ago, a cardiologist at Houston Methodist Hospital suggested he see an adult congenital heart defect specialist, who told him the repair surgeons made three decades earlier wasn't as permanent as he had thought. Efforts to enlarge a narrow pulmonary valve during his pediatric surgery had left the valve leaky, causing the right side of his heart to enlarge to massive size. Without a second procedure, his heart ultimately would fail.

Progress in saving kids with heart defects has led to a growing population of adults with repaired hearts that may not stand the test of time. As they live into their 30s, 40s and beyond, many will need second or third surgeries to address the consequences of their initial repairs.

Yet surveys show few adult survivors or even their doctors know of their increased risk of complications. Experts say such patients need specialized oversight and care, but the health care system is not set up to handle their needs.

"We really need to get them in before something bad happens to them," said Dr. C. Huie Lin, an adult congenital heart defect specialist at Houston Methodist.

1944 breakthrough

Prior to the 1940s, children with cardiac birth defects generally died as infants. Then in 1944, a team of surgeons at Johns Hopkins, including Houston cardiac surgeon Dr. Denton Cooley, performed the first operation to fix the type of defect that affected Pharr. The invention of the heart bypass pump in the 1950s cleared the way for more complex repairs. By the 1960s and 1970s, hundreds of children were surviving each year. Many began to outlive the lifespan of their repair.

A recent study in Quebec shows how much life expectancy has increased. The study found the median age of death for those with congenital heart disease had increased from 60 in the 1987 to 1993 period to 75 from 1999 to 2005. For severe defects, the median age of death increased from 2 to 23. In the late 1980s, more than 25 percent of deaths occurred within the first year of life. By 2005, that rate was driven down to less than 5 percent.

Now the number of hospitalizations of adults with congenital heart disease is increasing twice as quickly as hospitalizations of children.

"They're going to take over soon," Lin said. "The reality is we have not had a handle on how successful the repairs have been. It's going to be a tidal wave."

There are an estimated 1.5 million adult survivors in the United States, with numbers growing about 5 percent per year and not nearly enough specialists to see them. Houston, for example, has about 40 pediatric cardiologists, and Texas Children's Hospital performs 800 to 900 cardiac surgeries each year. However, there are only seven adult congenital heart defect specialists in Houston to treat an estimated 30,000 patients.

"Most of the time, they get lost to follow-up or they don't get referred to adult congenital specialists, or they're just not seeing anybody," Lin said.

Tetralogy of Fallot

Pharr's defect was a set of four problems known collectively as Tetralogy of Fallot: a hole in the wall between the left and right ventricles, a right heart chamber with walls that are too thick, a misplaced aorta, and a narrow valve between the heart and lungs.

Doctors have developed techniques to repair the hole in the heart wall and to open up the narrow heart valve to the lungs. As part of that valve repair, surgeons usually stretch the valve between the heart and lung, which almost invariably causes the valve to leak.

For years, doctors thought the leak wouldn't pose a problem, but over time they've learned the leaky valve can cause the right side of the heart to get bigger, affecting its pumping strength.

With the right side of his heart expanding to a dangerous size, Pharr had his pulmonary valve replaced at age 33 and later had additional procedures to correct an abnormal heart rhythm.

"The problem is we need to get early and preventive care done," Lin said. "If you wait until things get catastrophic, you take a $100,000 surgery and turn it into a $1 million, $2 million or $5 million hospitalization."

A 2010 study by the Adult Congenital Heart Association concluded more than half of parents of children with congenital heart disease did not know their child would need lifelong care.

"For a long time, when a baby was born with complex congenital heart disease, we said to the parents, 'The bad news is your kid has congenital heart disease. The good news is we can fix it,'" said Sue Fernandes, director of the Adult Congenital Heart Program at Lucile Packard Children's Hospital and Stanford Hospital and Clinics in Palo Alto, Calif.

'A bit of denial'

Doctors now have typically stopped calling it a cure or a fix, instead saying each step could buy the patient another 10 to 20 years.

"If you go through that four times, you're going to be great," Lin said.

But a recent survey of nearly 300 teens with congenital heart defects found nearly identical results. Patients didn't know about their need for follow-up.

"There's a bit of denial, too," Lin said. "If you spend a month in the hospital as a kid, you really don't want to go back."

A study published in March by doctors at The Children's Hospital of Philadelphia found one in five adults living with congenital heart defects showed signs of post-traumatic stress disorder. Patients live in fear that a routine exam will reveal another problem, forcing them to have another major operation.

"My worst nightmare was to have another surgery," said Allison Bennie, a 28-year-old woman from South Carolina, born with Tetralogy of Fallot.

Bennie's family are Jehovah's Witnesses, whose faith forbids the use of blood transfusions. In 1988, her mother took her to Houston, where Cooley had agreed to do a bloodless surgery to address the defect. She hadn't seen a cardiologist again until 2010.

"My parents really didn't tell me a whole lot," she said. "I guess they in their own way were trying to protect me, but they assumed I wasn't going to have any issues later in life."

She returned to Houston in 2014. Lin and other surgeons were able to replace her valve in another bloodless surgery.

Treating congenital heart defects as more of a chronic disease than an acute problem makes the limited supply of specialists an even greater problem.

Even something as simple as an electrocardiogram reading, he said, can mean different things to a standard cardiologist and to one trained in congenital heart disease. Many patients continue to see their pediatric cardiologists well into adulthood. These doctors often are reluctant to refer patients to adult cardiologists given the lack of specialists, and many of the follow-up repairs are done at pediatric hospitals.

That becomes problematic when patients develop heart problems that come with aging, such as clogged arteries. An adult survivor in need of a valve replacement also might require a coronary bypass.

"I don't think you want to be doing that in a pediatric hospital," Fernandes said.

More recently, patients have had difficulties accessing specialty care due to the narrow health provider networks of their insurance plans.

"There's not the awareness from insurance plans and policymakers that it's not OK for them to go to a general cardiologist up the street," Fernandes said. "They need to go to a specialist."

Consistent tests

Guidelines call for adult survivors to get certain tests every three to five years. Some may need procedures or exercise testing. Patients might have a higher risk of cancer, due to the radiation exposure during treatment.

"We had never thought that some of these really complex patients would live long enough to develop acquired heart disease," Fernandes said. "If you were born with half a heart, we were just happy to get you to live to adulthood. Now most of our patients are doing well into their 40s."

Until recently, there had been no certification for adult congenital heart disease specialists and no accreditation of programs. The first group of physicians to be board-certified in adult congenital heart disease completed their certification exams last fall.

And the Adult Congenital Heart Association has launched a pilot for five programs to be accredited as adult congenital heart disease centers. With time, the accreditation could be expanded to the more than 100 hospital programs nationwide offering such services.

Advocates hope that once board certification and accreditation are widespread, the cost savings that come with properly managing such patients will be evident.

"It's very expensive, but we do feel ... it will be financially advantageous to stay on top of it and it doesn't cost as much later," Fernandes said. "We just don't have much data to back it up."

Reassuring presence

Pharr regularly visits Texas Children's Hospital to talk to patients going through their first or second heart surgery and their parents. A massive hulk of a man, he reassures many of the families that their frail children can live full, active lives.

On one of his rounds earlier this year, he encountered a 7-year-old boy crying and upset after doctors told him he needed a second surgery. Pharr pulled down his shirt collar, revealing the scar on his chest.

"You have a scar like me," the boy said. "How many surgeries have you had?"